Pelvic lipomatosis (PL) involves nonmalignant excess fat growth in perirectal and perivesical areas, gaining recognition over three decades. This review addresses its clinicopathological aspects amid controversies, emphasizing the need for a comprehensive examination of current literature. This report outlines a 42-year-old man’s diagnostic journey for persistent urological symptoms, ultimately identified as PL. PL, if untreated, adversely impacts the urinary system, affecting kidney function and causing systemic complications. Management involves accurate diagnosis through radiological methods and surgical intervention, aiming to alleviate symptoms and enhance affected individuals’ quality of life.